Publications by authors named "Annie Dionne"

Objective: The corticospinal tract (CST) reveals progressive microstructural alterations in ALS measurable by DTI. The aim of this study was to evaluate fractional anisotropy (FA) along the CST as a longitudinal marker of disease progression in ALS.

Methods: The study cohort consisted of 114 patients with ALS and 110 healthy controls from the second prospective, longitudinal, multicentre study of the Canadian ALS Neuroimaging Consortium (CALSNIC-2).

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Article Synopsis
  • This study explored how brain degeneration correlates with the progression of amyotrophic lateral sclerosis (ALS) by analyzing brain scans of 149 ALS patients and 144 healthy controls.
  • Researchers used a texture analysis method on MRI scans to identify "autocorrelation," a biomarker indicating brain degeneration, focusing on different stages of the disease.
  • Results showed that brain changes varied significantly depending on the criteria used for patient classification, suggesting that standard clinical trial measures may not fully capture the extent of brain degeneration in ALS patients.
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Background: Increased morbidity in many patients with myasthenia gravis (MG) on long-term immunosuppression highlights the need for improved treatments. The aim of this study is to investigate the safety and efficacy of iscalimab (CFZ533), a fully human anti-CD40 monoclonal antibody, in patients with moderate-to-severe MG receiving standard-of-care (SoC) therapies.

Methods: In this double-blind, placebo-controlled phase 2 study, symptomatic patients (n = 44) despite SoC were randomized 1:1 to receive intravenous iscalimab (10 mg/kg; n = 22) or placebo (n = 22) every 4 weeks for 6 doses in total.

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Amyotrophic Lateral Sclerosis (ALS) is a complex neurodegenerative disorder characterized by motor neuron degeneration. Significant research has begun to establish brain magnetic resonance imaging (MRI) as a potential biomarker to diagnose and monitor the state of the disease. Deep learning has emerged as a prominent class of machine learning algorithms in computer vision and has shown successful applications in various medical image analysis tasks.

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Purpose: The primary objective of this study was to determine if speech and pause measures obtained using a passage reading task and timing measures from a monosyllabic diadochokinesis (DDK) task differ across speakers of Canadian French diagnosed with amyotrophic lateral sclerosis (ALS) presenting with and without bulbar symptoms, and healthy controls. The secondary objective was to determine if these measures can reflect the severity of bulbar symptoms.

Method: A total of 29 Canadian French speakers with ALS (classified as bulbar symptomatic [ = 14] or pre-symptomatic [ = 15]) and 17 age-matched healthy controls completed a passage reading task and a monosyllabic DDK task (/pa/ and /ta/), for up to three follow-up visits.

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Although speech declines rapidly in some individuals with amyotrophic lateral sclerosis (ALS), longitudinal changes in speech have rarely been characterized. The study objectives were to model the rate of decline in speaking rate and speech intelligibility as a function of disease onset site, sex, and age at onset in 166 individuals with ALS; and estimate time to speech loss from symptom onset. We also examined the association between clinical (speaking rate/intelligibility) measures and patient-reported measures of ALS progression (ALSFRS-R).

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Amyotrophic lateral sclerosis (ALS) is a multisystem neurodegenerative disorder characterized by progressive degeneration of upper motor neurons and lower motor neurons, and frontotemporal regions resulting in impaired bulbar, limb, and cognitive function. Magnetic resonance imaging studies have reported cortical and subcortical brain involvement in the pathophysiology of ALS. The present study investigates the functional integrity of resting-state networks (RSNs) and their importance in ALS.

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: Amyotrophic lateral sclerosis (ALS) is a multi-system disorder characterized primarily by motor neuron degeneration, but may be accompanied by cognitive dysfunction. Statistically appropriate criteria for establishing cognitive impairment (CI) in ALS are lacking. We evaluate quantile regression (QR), that accounts for age and education, relative to a traditional two standard deviation (SD) cutoff for defining CI.

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The absence of disease modifying treatments for amyotrophic lateral sclerosis (ALS) is in large part a consequence of its complexity and heterogeneity. Deep clinical and biological phenotyping of people living with ALS would assist in the development of effective treatments and target specific biomarkers to monitor disease progression and inform on treatment efficacy. The objective of this paper is to present the Comprehensive Analysis Platform To Understand Remedy and Eliminate ALS (CAPTURE ALS), an open and translational platform for the scientific community currently in development.

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Progressive cerebral degeneration in amyotrophic lateral sclerosis (ALS) remains poorly understood. Here, three-dimensional (3D) texture analysis was used to study longitudinal gray and white matter cerebral degeneration in ALS from routine T1-weighted magnetic resonance imaging (MRI). Participants were included from the Canadian ALS Neuroimaging Consortium (CALSNIC) who underwent up to three clinical assessments and MRI at four-month intervals, up to 8 months after baseline (T ).

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Article Synopsis
  • A study was conducted to assess genetic testing practices at ALS clinics across Canada, using surveys and interviews with clinicians from 27 clinics.
  • Results showed that 93% of clinics regularly test for familial ALS, but only 33% do so for sporadic ALS, highlighting significant barriers such as treatment impact perception, approval challenges, and inadequate genetic counseling access.
  • Findings indicate that genetic testing access varies widely, with an average wait time of 10 months to see a genetic counselor, and calls for improved testing practices to ensure equitable healthcare delivery for ALS patients.
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Article Synopsis
  • A study was conducted to assess the safety, dose response, and initial efficacy of a treatment for patients with amyotrophic lateral sclerosis (ALS) over 12 weeks.
  • A total of 458 patients were enrolled, with results showing no significant improvements in the primary endpoint of slow upright vital capacity (SVC) or secondary measures like the ALS Functional Rating Scale and muscle strength mega-score during the 12 weeks.
  • While the main analysis did not reach statistical significance, trends indicated potential benefits for active treatment compared to placebo, and the treatment was generally well tolerated, with minor side effects and reversible liver and kidney issues noted.
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The spinal muscular atrophies (SMA) affect lower motor neurons leading to important muscle atrophy and paralysis. Some cases of SMA affect mostly the lower limbs and are called autosomal dominant spinal muscular atrophy, lower extremity predominant (SMALED). So far, two genes have been identified to cause this phenotype, DYNC1H1 (SMALED1) and BICD2 (SMALED2).

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Loss-of-function mutations in the potassium-chloride cotransporter KCC3 lead to Andermann syndrome, a severe sensorimotor neuropathy characterized by areflexia, amyotrophy and locomotor abnormalities. The molecular events responsible for axonal loss remain poorly understood. Here, we establish that global or neuron-specific KCC3 loss-of-function in mice leads to early neuromuscular junction (NMJ) abnormalities and muscular atrophy that are consistent with the pre-synaptic neurotransmission defects observed in patients.

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Introduction: In recent studies on ultrasound-guided infraclavicular block (ICB), the authors have favoured a single injection posterior to the axillary artery rather than multiple injections; however, procedural complications and success rates associated with single-injection ultrasound-guided ICB are not well known. We undertook an observational study to evaluate the success rates of experienced and non-experienced operators performing ICBs and to identify the complications associated with ultrasound-guided single-injection ICB.

Methods: We conducted an observational cohort study of all ultrasound-guided single-injection ICBs performed over a two-year period (2008-2010).

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Up to 16% of chronic inflammatory demyelinating polyneuropathy (CIDP) patients may present acutely. We performed a retrospective chart review on 30 acute inflammatory demyelinating polyneuropathy (AIDP) and 15 acute-onset CIDP (A-CIDP) patients looking for any clinical or electrophysiological parameters that might differentiate AIDP from acutely presenting CIDP. A-CIDP patients were significantly more likely to have prominent sensory signs.

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Background: This study aims to validate the fitting of contact lenses derived from refractive and keratometric values in a group of subjects with moderate (2.50 diopters [D] to 5.00 D) to severe (>5.

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To determine the optimal recording site for phrenic nerve conduction studies, six different recording techniques were compared in 11 healthy volunteers (22 phrenic nerves). The mean diaphragm compound muscle action potential (CMAP) amplitude, side-to-side difference, and the number of studies with a false-positive result (CMAP amplitude <0.30 mV) were compared for each technique.

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We present a case of subacute weakness of one hand with unusual sensory involvement including the upper thorax. Despite normal distal conduction studies, a clinical diagnosis of Lewis-Sumner syndrome was made and the patient responded well to intravenous immunoglobulins. Repeated studies after clinical exacerbation finally proved the demyelinating nature of the neuropathy using proximal magnetic nerve stimulation.

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Background: X-linked adrenoleukodystrophy is a peroxisomial disorder caused by mutations in the ABCD1 gene. Adrenomyeloneuropathy is the second most frequent phenotype (25-46%) of this disease and classically presents in adulthood with spastic paraparesis. Female heterozygotes can be symptomatic, but they are frequently misdiagnosed as having multiple sclerosis.

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